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Factor X activation deficiency

MedGen UID:
870255
Concept ID:
C4024693
Finding
HPO: HP:0008354

Definition

Reduced ability to transform factor X into its activated form factor Xa. [from HPO]

Term Hierarchy

Conditions with this feature

SCOTT SYNDROME
MedGen UID:
167107
Concept ID:
C0796149
Disease or Syndrome
Scott syndrome (SCTS) is a mild platelet-type bleeding disorder characterized by impaired surface exposure of procoagulant phosphatidylserine (PS) on platelets and other blood cells, following activation with Ca(2+)-elevating agents (Munnix et al., 2003).

Professional guidelines

PubMed

Shapiro A
Thromb Res 2020 Dec;196:590-602. Epub 2019 Jul 19 doi: 10.1016/j.thromres.2019.07.014. PMID: 31420204
Dorgalaleh A, Alavi SE, Tabibian S, Soori S, Moradi E, Bamedi T, Asadi M, Jalalvand M, Shamsizadeh M
Hematology 2017 May;22(4):224-230. Epub 2016 Nov 28 doi: 10.1080/10245332.2016.1263007. PMID: 27894217
Ilonczai P, Oláh Z, Selmeczi A, Kerényi A, Bereczky Z, Póka R, Schlammadinger Á, Boda Z
Blood Coagul Fibrinolysis 2015 Oct;26(7):798-804. doi: 10.1097/MBC.0000000000000348. PMID: 26226254

Recent clinical studies

Etiology

Wang J, Keshava S, Das K, Kolesnick R, Jiang XC, Pendurthi UR, Rao LVM
Arterioscler Thromb Vasc Biol 2023 Jan;43(1):64-78. Epub 2022 Nov 22 doi: 10.1161/ATVBAHA.122.318443. PMID: 36412194Free PMC Article
Gao YJ, Shen KN, Chang L, Feng J, Mao YY, Zhang L, Cao XX, Zhou DB, Li J
Leuk Res 2022 Sep;120:106917. Epub 2022 Jul 5 doi: 10.1016/j.leukres.2022.106917. PMID: 35849875
Shapiro A
Thromb Res 2020 Dec;196:590-602. Epub 2019 Jul 19 doi: 10.1016/j.thromres.2019.07.014. PMID: 31420204
Oldenburg J, Mahlangu JN, Kim B, Schmitt C, Callaghan MU, Young G, Santagostino E, Kruse-Jarres R, Negrier C, Kessler C, Valente N, Asikanius E, Levy GG, Windyga J, Shima M
N Engl J Med 2017 Aug 31;377(9):809-818. Epub 2017 Jul 10 doi: 10.1056/NEJMoa1703068. PMID: 28691557
Menegatti M, Peyvandi F
Semin Thromb Hemost 2009 Jun;35(4):407-15. Epub 2009 Jul 13 doi: 10.1055/s-0029-1225763. PMID: 19598069

Diagnosis

Nanjo S, Wu W, Karachaliou N, Blakely CM, Suzuki J, Chou YT, Ali SM, Kerr DL, Olivas VR, Shue J, Rotow J, Mayekar MK, Haderk F, Chatterjee N, Urisman A, Yeo JC, Skanderup AJ, Tan AC, Tam WL, Arrieta O, Hosomichi K, Nishiyama A, Yano S, Kirichok Y, Tan DS, Rosell R, Okimoto RA, Bivona TG
J Clin Invest 2022 Jul 1;132(13) doi: 10.1172/JCI145099. PMID: 35579943Free PMC Article
Moore GW
Semin Thromb Hemost 2022 Mar;48(2):145-160. Epub 2021 Aug 12 doi: 10.1055/s-0041-1732465. PMID: 34384127
Link KG, Stobb MT, Monroe DM, Fogelson AL, Neeves KB, Sindi SS, Leiderman K
Arterioscler Thromb Vasc Biol 2021 Jan;41(1):79-86. Epub 2020 Oct 29 doi: 10.1161/ATVBAHA.120.314648. PMID: 33115272Free PMC Article
Dorgalaleh A, Alavi SE, Tabibian S, Soori S, Moradi E, Bamedi T, Asadi M, Jalalvand M, Shamsizadeh M
Hematology 2017 May;22(4):224-230. Epub 2016 Nov 28 doi: 10.1080/10245332.2016.1263007. PMID: 27894217
Menegatti M, Peyvandi F
Semin Thromb Hemost 2009 Jun;35(4):407-15. Epub 2009 Jul 13 doi: 10.1055/s-0029-1225763. PMID: 19598069

Therapy

Nanjo S, Wu W, Karachaliou N, Blakely CM, Suzuki J, Chou YT, Ali SM, Kerr DL, Olivas VR, Shue J, Rotow J, Mayekar MK, Haderk F, Chatterjee N, Urisman A, Yeo JC, Skanderup AJ, Tan AC, Tam WL, Arrieta O, Hosomichi K, Nishiyama A, Yano S, Kirichok Y, Tan DS, Rosell R, Okimoto RA, Bivona TG
J Clin Invest 2022 Jul 1;132(13) doi: 10.1172/JCI145099. PMID: 35579943Free PMC Article
Oe Y, Miyazaki M, Takahashi N
Tohoku J Exp Med 2021 Sep;255(1):1-8. doi: 10.1620/tjem.255.1. PMID: 34511578
Østergaard H, Lund J, Greisen PJ, Kjellev S, Henriksen A, Lorenzen N, Johansson E, Røder G, Rasch MG, Johnsen LB, Egebjerg T, Lund S, Rahbek-Nielsen H, Gandhi PS, Lamberth K, Loftager M, Andersen LM, Bonde AC, Stavenuiter F, Madsen DE, Li X, Holm TL, Ley CD, Thygesen P, Zhu H, Zhou R, Thorn K, Yang Z, Hermit MB, Bjelke JR, Hansen BG, Hilden I
Blood 2021 Oct 7;138(14):1258-1268. doi: 10.1182/blood.2020010331. PMID: 34077951Free PMC Article
Oldenburg J, Mahlangu JN, Kim B, Schmitt C, Callaghan MU, Young G, Santagostino E, Kruse-Jarres R, Negrier C, Kessler C, Valente N, Asikanius E, Levy GG, Windyga J, Shima M
N Engl J Med 2017 Aug 31;377(9):809-818. Epub 2017 Jul 10 doi: 10.1056/NEJMoa1703068. PMID: 28691557
Menegatti M, Peyvandi F
Semin Thromb Hemost 2009 Jun;35(4):407-15. Epub 2009 Jul 13 doi: 10.1055/s-0029-1225763. PMID: 19598069

Prognosis

Oe Y, Miyazaki M, Takahashi N
Tohoku J Exp Med 2021 Sep;255(1):1-8. doi: 10.1620/tjem.255.1. PMID: 34511578
Kitazawa T, Shima M
Int J Hematol 2020 Jan;111(1):20-30. Epub 2018 Oct 22 doi: 10.1007/s12185-018-2545-9. PMID: 30350119
Pavlova A, Preisler B, Driesen J, de Moerloose P, Zieger B, Hütker S, Dengler K, Harbrecht U, Oldenburg J
Haemophilia 2015 May;21(3):386-391. Epub 2015 Jan 13 doi: 10.1111/hae.12604. PMID: 25582404
Chen X, Iliopoulos D, Zhang Q, Tang Q, Greenblatt MB, Hatziapostolou M, Lim E, Tam WL, Ni M, Chen Y, Mai J, Shen H, Hu DZ, Adoro S, Hu B, Song M, Tan C, Landis MD, Ferrari M, Shin SJ, Brown M, Chang JC, Liu XS, Glimcher LH
Nature 2014 Apr 3;508(7494):103-107. Epub 2014 Mar 23 doi: 10.1038/nature13119. PMID: 24670641Free PMC Article
Brown DL, Kouides PA
Haemophilia 2008 Nov;14(6):1176-82. doi: 10.1111/j.1365-2516.2008.01856.x. PMID: 19141158

Clinical prediction guides

Gao YJ, Shen KN, Chang L, Feng J, Mao YY, Zhang L, Cao XX, Zhou DB, Li J
Leuk Res 2022 Sep;120:106917. Epub 2022 Jul 5 doi: 10.1016/j.leukres.2022.106917. PMID: 35849875
Østergaard H, Lund J, Greisen PJ, Kjellev S, Henriksen A, Lorenzen N, Johansson E, Røder G, Rasch MG, Johnsen LB, Egebjerg T, Lund S, Rahbek-Nielsen H, Gandhi PS, Lamberth K, Loftager M, Andersen LM, Bonde AC, Stavenuiter F, Madsen DE, Li X, Holm TL, Ley CD, Thygesen P, Zhu H, Zhou R, Thorn K, Yang Z, Hermit MB, Bjelke JR, Hansen BG, Hilden I
Blood 2021 Oct 7;138(14):1258-1268. doi: 10.1182/blood.2020010331. PMID: 34077951Free PMC Article
Link KG, Stobb MT, Monroe DM, Fogelson AL, Neeves KB, Sindi SS, Leiderman K
Arterioscler Thromb Vasc Biol 2021 Jan;41(1):79-86. Epub 2020 Oct 29 doi: 10.1161/ATVBAHA.120.314648. PMID: 33115272Free PMC Article
Shapiro A
Thromb Res 2020 Dec;196:590-602. Epub 2019 Jul 19 doi: 10.1016/j.thromres.2019.07.014. PMID: 31420204
Esmon CT
Prog Hemost Thromb 1984;7:25-54. PMID: 6099583

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